Let’s shift gear and talk a little
bit about choledochal cysts.
Choledochal cysts are a
that cause abnormal
enlargement of the bile duct.
This causes obstruction and hepatic
congestion and eventually liver failure.
It’s not that uncommon.
You can see here a picture of
a patient with a gallbladder
and a large excised
It happens about 1:100,000
to 1:150,000 patients.
So you’ll see one every several years
in a large birthing institution.
There are several
different types of cysts.
I don’t think it’s
likely that the test is
going to quiz you on
which type of cyst it is
but it is important to remember
that there are different types,
and so elucidating what the type is
may be beneficial in terms of understanding
exactly what’s going on with that patient.
By far the most common is a cystic
dilatation of the extrahepatic biliary duct,
which you can see here.
You can here on your slide is a normal
biliary tree and then next to it,
in that common bile duct,
there’s just an outpouching
or cyst that’s formed
along that duct.
Additionally, patients can have an abnormal
pouch or a sac opening from the duct,
this is a type 2.
A type 3 is a cyst, which is located
within the wall of the actual duodenum.
A type 4 is when you have
swellings both in the
extrahepatic biliary tracts,
so it’s inside the liver
and just outside it.
And type 5 is when there are
multiple intrahepatic cysts.
This is the least common type.
So key symptoms for any
child with choledochal cyst
is typically they will have
right upper quadrant pain.
Now, that’s on the test.
It’s sometimes hard to truly determine
where the quadrant pain is in an infant,
but you should see it.
But key -- key and highly likely
to show up on your test --
these children will
and they will have
So they’ll have acholic stools again because
that bile isn’t getting into the stools
and their jaundice will be from the
hyperbilirubinemia, which is conjugated.
Often, they may have fever or infection as
a result of these cysts becoming infected.
They may develop pancreatitis
and they often will have a palpable
mass in the right upper quadrant.
You may be able to palpate this cyst.
In general, we’ll get some labs.
We’ll get bilirubin,
which should be elevated
and should have a high
These patients will often have
elevated alk phos, AST, ALT,
and a high GGT as well because again
the biliary tree is involved.
As in other things, we’ll get a
coagulation profile, a PT/PTT,
to assess the function of the liver
and see how badly off they’re doing.
And a CBC is often
obtained as well.
So the definitive diagnosis though is
made through an abdominal ultrasound.
These cysts show up very nicely
on abdominal ultrasound.
Rarely, we may get abdominal CT,
maybe an MRI instead.
The MRI has the risk
of needing sedation.
The CT is a little bit quicker,
but has the risk of radiation.
Most centers are moving
more towards ultrasound.
However, sometimes the surgeons
want a really good idea
of what kind of choledochal cyst
this is that they’re dealing with
and so sometimes we’ll get a magnetic resonance
choleangiopancreatography or an MRCP
to try and get a sense of exactly what is
the structure, which type is this,
so the surgeons have an idea
of what they’re getting into.
Treatment of the choledochal
cyst is a complete excision
and a construction of a
biliary enteric anastomosis.
Sometimes it’s Kasai procedure.
It really depends on
what the problem is
and this is what the surgeons are going
to look into after the imaging is done.
So that is an assessment of
the major kinds of causes of
in young children.
Thanks for your time.